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Pediatric Hematological Diseases
Hemophilia
Hemophilia (Factor VIII deficiency)
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Sex linked recessive trait
Excessive bleeding after circumcision
Soft tissue bleeds
Joints swollen, warm, and painful
May have severe bleeding in GI tract,
peritoneal cavity or CNS
PTT is prolonged
DDAVP
Administer Factor VIII even with minor bleed
episodes
Gene Therapy
PTT is prolonged
• APTT
prolonged
Factor VIII deficiency
Factor VIII
• Blood product
• Diluted and given IV
The school nurse is assessing a child with
hemophilia who has pain in the knee. The
nurse suspects hem arthrosis. The nurse
should:
1. Perform range of motion
2. Elevate leg above the heart
3. Give ASA or ibuprophen for pain
4. Apply warm soaks to reduce
swelling
The nurse is giving factor viii to a child with
hemophilia. The nurse should observe for
which potential complication during the
infusion?
Fluid overload
Transfusion reaction
Emboli formation
Contracting AIDS
Von Will brand Disease
• Hereditary
• Plasma
• Symptoms:
• Diagnosis:
• Treatment:
Von Will brand factor
Idiopathic Thrombocytopenia Purpura (ITP)
• Autoimmune disorder characterized by
increased destruction of platelets
• Most common bleeding disorder in
children
• Most commonly 2-10 years age
• Usually follows viral infection
• S/S: multiple ecchymosis and petechial
• Diag: Hx, < platelets
• RX: steroids, IVIG
ITP
Purpura
Platelets
A child with a platelet dysfunction has been
receiving intravenous fluids for two days. The
nurse is discontinuing the IV.
The nurse should:
1. Restrict movement of the arm for
12 hours
2. Obtain a culture of the catheter tip
3. Place steri strips over the site
4. Apply direct pressure over the site
for 5 minutes
Sickle Cell Anemia
• Hereditary hemoglobinopathy
• Partial or complete replacement of
normal hemoglobin S
• Any organ can be affected resulting in
organ related symptoms
Sickle cell anemia
• Inherited, autosomal recessive disease
• Affects RBC’s acutely sickle shaped
• 1 in 500 Black infants in US, occasionally
Mediterranean decent
• s/s not seen in newborn period due to high
levels of fetal hemoglobin
Symptoms in SCD
• Any organ can be affected and cause
symptoms
• Symptoms triggered by infection, fever,
dehydration, low oxygen level,
emotional stress
Clinical Manifestations
Clinical symptoms are directly related to
shortened life span of blood cells.
Pathologic changes occur in most body
systems and result in multiple signs and
symptoms
Autosomal Recessive
Diagnostic testing
• SCD test thru mandatory screening in
most states
• Hbg electrophoresis- positive diagnosis
• Sickledex-Quick screens over 6 months
age when fetal hbg fallen
Normal Blood Cell
Sickle Cell
Clinical Management
• Teach families watch for symptoms infection
• Careful hydration, pain management, oxygen, bed
rest to reduce energy and 02 expenditure
• Transfusions for severe anemia as needed
• Painful episodes—NSAIDS and narcotics
Complications of SCD
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Stroke (6-15%) CVA
Acute chest syndrome
Abdominal crises
Splenic Sequestration
Priapism (painful engorgement of penis)
Leg ulcers
Decreased resistance to infection
Review question SCD
Which of the following is true about
SCD?
A. Sickle cells carry excess oxygen
B. Sickle cells decrease blood
viscosity
C. Sickle cell anemia results in
decreased RBC production
D. Sickle cell anemia results in
increased RBC destruction
A two year old child is in SCA crisis. The child is
lying with knees flexed against abdomen
The nurse would assess further for:
1. Stomach pain
2. Nausea
3. Constipation
4. Fear 2ndary to hospitalization
Iron Deficiency Anemia
•Most common
childhood anemia
•Neonatal iron stores
from mother depleted
after 6 months
Causes of Iron Deficiency anemia
• Inadequate supply of iron in blood
• Excessive milk intake
• Inadequate iron stores at birth
• Impaired absorption
• Blood loss and helminthes
• Excessive demands for iron for growth
Clinical Manifestations
•Pallor, fatigue,
irritability
•Lab data: HGB,
MCH, MCV, serum
iron all decreased
Underweight
Underweight Undernourished
Clinical Manifestations
• Clubbing of fingers
Overweight
Diagnosis IDA
• Lab studies, Hgb, MCV, microscopic
analysis, serum iron binding capacity
• Diet history and analysis
Best food sources of iron
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Chicken liver, cooked
Crab pieces steamed
Beef Liver fried
Soybeans, baked
Spinach, cooked
Potato, baked
Scallops, steamed
Pistachios, dried
Broccoli, cooked
Cashews, dry roasted
Turkey, dark meat
Complications of Iron deficiency
• Behavior problems
• Impaired Cognition
• Social problems
• Research needed and ongoing
Nursing Management/Treatment
•Iron absorbed best on
empty stomach
•Teach developmental
nutrition
Nursing Management
• Absorption
• Vitamin C
• Dental Care
• Tarry stools
• GI irritation
Nursing Management of IDA
• Screening 9 months
• Plot growth and development
• H and H—more detailed tests if
abnormal
• Diet History and education
Aplastic Anemia
• Deficiency of all blood cells
• Congenital
• Acquired
Aplastic Anemia
Disseminated Intravascular Coagulation
• Serious condition in which clotting
mechanisms are disturbed
• Leads to extensive clotting and
tissue damage.
Review Questions
• Foods that contain iron for a teenager’s
diet are?
• How is iron best absorbed?
• What vitamin helps absorption of iron?
• Side effects of iron taken orally?
Questions
Immune Function
In Children
Severe Combined Immune Deficiency Disorder
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Etiology
Clinical Manifestations
Diagnosis
Clinical Therapy
Prognosis
Nursing Management
Wiskott-Aldrich Syndrome
• Combined congenital immune
deficiency syndrome
• X linked recessive
• 4 in 1 million male births
• Diagnosis: Thrombocytopenia
• Treatment
• Nursing Management
HIV follows two patterns in children
• Fast progresses—about 1/3 of children
who develop serious signs and symptoms
including encephalopathy, FTT, and
opportunistic infections within the first 2
years of life
• Less rapid progresses—approximately
2/3’s of children who develop less serious
symptoms such as lymphadenopathy and
recurrent childhood illness
Diagnostic testing
• HIV PCR (polymerase chain reaction)
done by 2 weeks of age, preferable in
first 48 hrs and repeated in 2 weeks if
neg-also at 1-2-3-6 months
• Antigen antibody ELISA ^ 18 months
• CD4: measure of how well immune
system working
• Viral load: measure how much virus
in blood
Treatment HIV
• Pregnant HIV women should start ZDV 1434 weeks thru pregnancy
• Imperative women in labor to get ZDV for
babies to receive it in first 12 hrs life
• Babies ZDV 4x day until 6 weeks old
• Exposed children followed closely treated
with antivirals—method ongoing and
effective
• Monitor viral load, labs—very supportive
care for entire family
Immunizations and HIV
• Same immunizations on same schedule as
other children, including IPV, should not
receive OPV
• May receive MMR and Varicella vaccines if
asymptomatic and CD4 levels 15-25 %
• If exposed to Varicella and did not receive
vaccine should be given VCG
• Should take Pneumococcal and Influenza
vaccines
Review
• What is the difference between HIV and
AIDS?
• What vaccines should a child with HIV
receive at the 2 year visit (if the CD4 count
is normal)?
• What if a child with HIV is exposed to C
Pox and has not had the immunization?
What should the child receive?
Herpes simplex 1 more than 1 mo.
Herpes simplex 1 eye
Kaposi's sarcoma
Oral Kaposi sarcoma
Pneumocystis Carinii Pneumonia (PCP)
Juvenile Rheumatoid Arthritis
• Chronic joint
inflammation
diagnosed prior to
16 yrs age
• Peak age 1-3 yrs
• > in females
• Systemic
autoimmune
disorder with multiple
manifestations
Juvenile idiopathic Arthritis
• Polyarthritis:5 or more joints and
uveitis (30%)
• Oligo arthritis: knees, ankles, elbows
affects females (50-60%)
• Systemic: males and females+ many
joints, fever, rash, lymphadenopathy,
hepatic splenomegaly
Etiology/Prognosis
• Theories of autoimmune process
• 70 % experience permanent
remission of disease by adulthood
• Early onset better prognosis for
complete recovery
Pathophysiology/ Clinical S/S of JRA
• Chronic
inflammation
of synovium with
joint
effusion and eventual
erosion, destruction,
fibrosis or articular
cartilage
• Symptoms
Diagnosis and Management JRA
• Diagnosis of
exclusion
• No specific labs
• RF, HLA B27, ANA
ESR, CRP detect
amount of
inflammation
• X-rays rule out
fractures
Management
• Medication, OT, PT, Exercise
(noncontact)
• Education :Complications Uveitis
Well balanced diet, Meds with food
Influenza vaccine yearly
Peer contact
Over exertion exacerbation symptoms
Review Study
• What would an appropriate activity
be for a child with JRA?
• What is thought to be the cause of
JRA?
• What are the common symptoms?
Review Study
• What would an appropriate activity
be for a child with JRA?
• What is thought to be the cause of
JRA?
• What are the common symptoms?
Know s/s allergy
• Get to medical care ASAP
• Teach importance epipen
• Which of the following assessment
findings aids in diagnosing (HIV)
human deficiency syndrome in
children?
• A. excessive weight gain
• B. arrhythmia
• C. Intermittent diarrhea
• D. Tolerance of feedings
• The primary goal of treatment for the
child with JRA is to maintain:
• A. joint function
• B. excessive weight gain
• C. Social isolation
• D. Skin breakdown
• What oral drugs are used for JRA?
Side effects?
• What drug has ASA besides ASA?